Original articlePediatric cardiacUse of Oral Budesonide in the Management of Protein-Losing Enteropathy After the Fontan Operation
Section snippets
Patient Population
Between January 2005 and May 2009, 9 patients who had the Fontan operation and active PLE were started on a regimen of oral CR-budesonide. Patient characteristics including cardiac findings on echocardiography and cardiac catheterization were recorded. Chart review was performed with recording of response to oral CR-budesonide, efficacy, and side effects. Diagnosis of PLE was based on the clinical findings of peripheral edema or ascites and confirmation of hypoalbuminemia on multiple samples
Patient Characteristics
Median age at performance of the Fontan operation was 2.7 years (range, 1.2 to 10.8). By nature of their congenital heart disease, the functioning systemic single ventricle was a left ventricle in 4 patients and a right ventricle in the other 5. Median duration between the Fontan operation and initial diagnosis of PLE was 4 years (range, 0.1 to 13.3). Types of Fontan operation included lateral tunnel (n = 6), atriopulmonary (n = 2), and extracardiac (n = 1). Four were fenestrated at initial
Comment
To date, our understanding of PLE after the Fontan operation remains limited; however, we have developed a conceptual framework that is proving effective for management. We postulate that PLE after the Fontan operation is based on a fundamental alteration in hemodynamics, inherent in the physiology of relatively low cardiac output and elevated systemic venous pressure, findings present to some degree in all subjects with a Fontan circulation. The combination of low cardiac output and elevated
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2018, Progress in Pediatric CardiologyCitation Excerpt :The symptoms of PB can be mitigated by using aerosolized tissue plasminogen activator which may break down cast material facilitating their expectoration; the use of inhaled bronchodilatator, mucolytics and steroid has been reported also [68]. Butesonide is reported to reduce the inflammatory status of the gut meliorating the loss of proteins [69]. Common therapeutic strategies are reducing the systemic venous pressure with aggressive pulmonary vasodilatator therapy, such as sildenafil or bosentan, treating the Fontan sequelae as the stenosis of the Fontan pathway and/or creating a fenestration (Fig. 4).
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